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危机对成年尼日利亚镰状细胞贫血患者血红蛋白F水平的影响。

Influence of crisis on haemoglobin F level in adult Nigerian sickle cell anaemia patients.

作者信息

Olatunji P O, Falusi A G, Esien E M

机构信息

Department of Haematology, University of Ilorin, Nigeria.

出版信息

Cent Afr J Med. 1992 Jun;38(6):242-6.

PMID:1382859
Abstract

Forty-six Nigerian adult sickle cell anaemia patients were investigated, each in sickle cell crisis and steady state. Forty-three patients had vaso-occlusive crisis while three had haemolytic episodes. Investigations included Packed Cell Volume (PVC), Reticulocyte count and Haemoglobin F estimation. PCV was carried out by the microhaematocrit method while the reticulocytes were counted manually. Haemoglobin F was estimated by the Alkali Denaturation Technique. There was significant anaemia (p < 0.05) and reticulocytosis (p < 0.0001) during the period of crisis compared to the steady state. There was no significant difference (p > 0.05) between HbF level in crisis and that in the steady state. In other words the previously documented increase in HbF during reticulocyte response did not take place in our model. Maybe a 'critical' level of reticulocytosis was not attained. It was also shown that vaso-occlusive crisis did not induce an increase in HbF level suggesting that HbF might be genetically determined at a constant low level throughout life in each of our patients.

摘要

对46名尼日利亚成年镰状细胞贫血患者进行了调查,每位患者分别处于镰状细胞危象期和病情稳定期。43名患者发生血管闭塞性危象,3名患者出现溶血发作。检查项目包括红细胞压积(PVC)、网织红细胞计数和血红蛋白F测定。红细胞压积采用微量血细胞比容法进行检测,网织红细胞通过手工计数。血红蛋白F采用碱变性技术进行测定。与病情稳定期相比,危象期存在显著贫血(p < 0.05)和网织红细胞增多(p < 0.0001)。危象期与稳定期的血红蛋白F水平无显著差异(p > 0.05)。换句话说,在我们的模型中,先前记录的网织红细胞反应期间血红蛋白F升高并未发生。也许未达到“临界”网织红细胞增多水平。研究还表明,血管闭塞性危象并未导致血红蛋白F水平升高,这表明在我们的每位患者中,血红蛋白F可能在一生中都由基因决定处于恒定的低水平。

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